Concept information
Preferred term
Endocardial Fibroelastosis
Type
-
Topical Descriptor
Broader concept
Narrower concepts
Entry terms
- Endomyocardial Fibroelastosis
Scope note
- A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.
In other languages
-
Finnish
-
cardiomyopathia congenita
-
endokardiell fibroelastos
-
fibroelastosis endocardialis
-
kongenital kardiomyopati
-
sydämen sisäkalvon fibroelastoosi
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synnynnäinen sydänlihassairaus
-
Swedish
URI
http://www.yso.fi/onto/mesh/D004695
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